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J Med Internet Res 2020 May 14. Epub 2020 May 14.

Division of Rheumatology, Department of Medicine, University of Pennsylvania, Philadelphia, US.

Background: The conduct of clinical trials in rare diseases utilizing the traditional centers of excellence approach remains challenging. Patient-based registries have been shown to be both feasible and valid in several other diseases.

Objective: This report outlines the clinical characteristics of a large internet registry cohort of participants with a self-reported diagnosis of granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA). Read More

Clin Exp Rheumatol 2020 Mar-Apr;38 Suppl 124(2):201-206. Epub 2020 May 22.

Rheumatology Unit, Department of Medicine-DIMED, University Hospital, Padova, Italy.

Objectives: In ANCA-associated vasculitis (AAV), renal relapses are cause of concern as they are unpredictable and predictors of end-stage renal disease (ESRD). We aimed to assess the frequency of major renal (MR) relapses in AAV and to identify independent base-line predictors.

Methods: We performed a retrospective monocentric observational cohort study of patients affected by granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and renal limited vasculitis (RLV), diagnosed from 2000 to 2019, and who achieved clinical remission defined as Birmingham Vasculitis Activity Index version 3 (BVASv3)=0 and/or clinical judgment. Read More

Ophthalmic Plast Reconstr Surg 2020 May 19. Epub 2020 May 19.

Division of Oculofacial Plastic & Orbital Surgery, Gavin Herbert Eye Institute, University of California Irvine, Irvine, California, U.S.A.

A 63 year-old man presented with 3 weeks of worsening, painless left-sided proptosis, ptosis, and diplopia. Examination of the affected eye revealed normal visual acuity, normal intraocular pressure in the presence of a shallow anterior chamber, grade 1 relative afferent pupillary defect, restricted motility in all directions of gaze, and 6 mm of proptosis. Ultrasound biomicroscopy revealed uveal effusions with anterior rotation of the ciliary body causing appositional angle closure. Read More

Arthritis Rheumatol 2020 May 18. Epub 2020 May 18.

Division of Rheumatology, Department of Biostatistics, Epidemiology, and Informatics, University of Pennsylvania, Philadelphia, PA, USA.

Objective: Cutaneous manifestations of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV), including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA), are poorly characterized. This study describes the dermatologic features of AAV and their association with systemic manifestations of vasculitis.

Methods: A cross-sectional study describing and comparing the cutaneous manifestations of AAV was performed using data from a large, international, collaborative effort to collect comprehensive clinical data on patients with vasculitis. Read More

Front Neurol 2020 28;11:269. Epub 2020 Apr 28.

Neuroscience Section, Department of Pathophysiology and Transplantation, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.

Microscopic polyangiitis (MPA) is a necrotizing vasculitis that affects predominantly small-sized vessels in many organ systems. The disease generally causes glomerulonephritis, pulmonary damage, arthritis, and neuropathy. An exclusive involvement of both central nervous system (CNS) and peripheral nervous system (PNS) is extremely rare. Read More

Rheumatology (Oxford) 2020 May;59(Supplement_3):iii42-iii50

Department of Rheumatology, Skåne University Hospital, Lund, Sweden.

ANCA-associated vasculitis (AAV) includes granulomatosis with polyangiitis, microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis. Epidemiological studies in AAV are important in understanding possible aetiologic mechanisms and facilitating healthcare planning. However, epidemiological studies present a number of challenges including clear definition of cases differentiated from other clinical disorders, and identification of cases due to the rarity of AAV. Read More

Rheumatology (Oxford) 2020 May;59(Supplement_3):iii60-iii67

Vasculitis.at, Vienna, Austria.

ANCA-associated vasculitis (AAV) is a systemic, potentially organ and life threatening chronic autoimmune disease. With current management strategies, such as high-dose glucocorticoids in combination with cyclophosphamide or rituximab, outcomes have progressively improved with overall remission rates of 70-90%. However, relapse rates after discontinuation of therapy are consistently high, and treatment-related toxicity, mainly driven by glucocorticoids, still determines morbidity and quality of life. Read More

J Autoimmun 2020 Apr 24:102467. Epub 2020 Apr 24.

Department of Internal Medicine, National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, APHP, Université Paris Descartes, Paris, France. Electronic address:

Objective: To describe characteristics and long-term outcomes of patients with microscopic polyangiitis (MPA), an antineutrophil cytoplasm antibody (ANCA)-associated small-vessel necrotizing vasculitis.

Methods: MPA patients from the French Vasculitis Study Group Registry satisfying the European Medicines Agency algorithm were analyzed retrospectively. Characteristics at diagnosis, treatments, relapses and deaths were analyzed to identify factors predictive of death or relapse. Read More

Clin Exp Rheumatol 2020 Mar-Apr;38 Suppl 124(2):221-231. Epub 2020 Apr 22.

Department of Medicine and Surgery, University of Milan Bicocca, Respiratory Unit, San Gerardo Hospital, ASST Monza, Italy.

Antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) is a group of systemic vasculitides that predominantly affect small vessels, including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). Pulmonary involvement is frequently observed in AAV patients, with various possible phenotypes in the different diseases. In the last years, among the possible types of lung involvement, a growing interest has been addressed to the interstitial lung disease (ILD). Read More

Rheumatol Int 2020 Jul 20;40(7):1089-1099. Epub 2020 Apr 20.

Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, Republic of Korea.

Current evidence suggests that high uric acid levels are associated with accelerated renal damage. However, the clinical impact of serum uric acid level on patients with microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) is unknown. We aimed to evaluate the impact of hyperuricemia on such patients. Read More

Case Rep Dermatol 2020 Jan-Apr;12(1):52-56. Epub 2020 Mar 20.

Department of Dermatology, Gifu University Graduate School of Medicine, Gifu, Japan.

Palisaded neutrophilic and granulomatous dermatitis (PNGD) shows various clinical features and is histologically characterized by palisaded granulomas surrounding degenerated collagen. PNGD is known to be associated with a variety of systemic conditions such as rheumatoid arthritis and systemic lupus erythematosus. Furthermore, PNGD has been reported to be associated with antineutrophilic cytoplasmic antibody-associated vasculitis, including granulomatosis with polyangiitis (GPA) and microscopic polyangiitis. Read More

Turk J Med Sci 2020 04 18. Epub 2020 Apr 18.

Background/aim: There is a need for a scoring system for predicting the overall and ICU prognosis of patients with ANCA-associated vasculitis (AAV), but there are limited data on it in the literature. Therefore, we aimed to determine the scores that can estimate the prognosis of patients with AAV during intensive care follow-up .

Materials And Methods: All adult patients admitted to the medical ICUs of four reference university hospitals in Turkey due to AAV activation and/or disease/treatment complications in the last ten years were included in this study. Read More

Int J Rheum Dis 2020 Apr 16. Epub 2020 Apr 16.

Department of Immunopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

Aim: Antineutrophil cytoplasmic antibody-associated vasculitis (AAV) is an autoimmune disease characterized by necrotizing small vessel vasculitis that can affect various organs and present multiple symptoms. Susceptibility to AAV is multifactorial and most likely caused by an amalgamation of genetic and environmental factors. The aim of the present study was to explore the distribution of human leukocyte antigen (HLA)-DRB1/DQB1, protein tyrosine phosphatase non-receptor type 22 (PTPN22) and cytotoxic T-Lymphocyte-associated protein 4 (CTLA-4) polymorphisms in North Indian AAV patients and their associations with clinical and pathological characteristics associated with the disease. Read More

Expert Opin Biol Ther 2020 May 5:1-12. Epub 2020 May 5.

Referral Center for Rare Systemic and Autoimmune Diseases, Department of Internal Medicine, Hôpital Cochin, Université Paris Descartes, Paris, France.

: Rituximab, an anti-B-cell biological therapy, has been investigated in several clinical trials on antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs).: In this paper, the clinical trials and open-label studies on rituximab efficacy and safety in treating AAVs are reviewed.: Rituximab achieved high remission-induction and sustained-maintenance rates for patients with these severe diseases, thereby challenging the cornerstone treatment of corticosteroids and cyclophosphamide followed by azathioprine. Read More

Scand J Rheumatol 2020 Apr 14:1-11. Epub 2020 Apr 14.

Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan.

: The complement cascade, especially the alternative pathway of complement, has been shown in basic research to be associated with anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV). We aimed to elucidate relationships between serum complement components and clinical characteristics in AAV.: In a nationwide prospective cohort study (RemIT-JAV-RPGN), we measured the serum levels of C1q, C2, C3, C3b/iC3b, C4, C4b, C5, C5a, C9, factor B, factor D, factor H, factor I, mannose-binding lectin, and properdin in 52 patients with microscopic polyangiitis (MPA) and 39 patients with granulomatosis with polyangiitis (GPA). Read More

Case Rep Nephrol 2020 26;2020:1426401. Epub 2020 Mar 26.

Penn Medicine Princeton Medical Center, Princeton Hypertension and Nephrology, 1 Plainsboro Rd, Plainsboro Township, NJ 08536, USA.

Microscopic polyangiitis (MPA) is an idiopathic autoimmune disease characterized by systemic vasculitis. While the lungs and kidneys are the major organs affected by MPA, it is known to involve multiple organ systems throughout the body. Temporal artery involvement is a very rare finding in MPA. Read More

Case Rep Dent 2020 14;2020:7020595. Epub 2020 Mar 14.

Department of Oral and Maxillofacial Surgery, Saitama Medical Center, Saitama Medical University, Saitama, Japan.

The antineutrophil cytoplasmic antibody- (ANCA-) associated vasculitides (AAVs), which include fever of unknown origin (FUO), are rare diseases characterized by necrotizing inflammation of small blood vessels and the presence of ANCAs. Microscopic polyangiitis (MPA) is a subtype of the AAVs. Although the prevalence of AAVs has generally increased over the last 20 years, there have been rare reports from the dental and oral surgery field. Read More

J Clin Rheumatol 2020 Mar 31. Epub 2020 Mar 31.

Division of Rheumatology, Mayo Clinic, Jacksonville, FL.

Background/objective: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) may involve the kidney, respiratory tract, skin, or central and peripheral nervous system. Reports of interstitial lung disease (ILD) in AAV (AAV-ILD) have been increasing.

Methods: We reviewed the medical records of all patients with AAV-ILD between January 1, 2007, and December 31, 2017, and compared their pulmonary involvement to patients with idiopathic pulmonary fibrosis (IPF). Read More

Autoimmun Rev 2020 Jun 29;19(6):102532. Epub 2020 Mar 29.

Priority Area Asthma & Allergy, Research Center Borstel, Airway Research Center North (ARCN), Members of the German Center for Lung Research (DZL), Borstel, Germany. Electronic address:

The human leukocytes antigen (HLA)-DRB1*16:02 allele has been suggested to be associated with many autoimmune diseases. However, a validation of the results of the different studies by a comprehensive analysis of the corresponding meta data is lacking. In this study, we performed a meta-analysis of the association between HLA-DRB1*16:02 allele with various autoimmune disorders. Read More

FASEB J 2020 May 30;34(5):6215-6228. Epub 2020 Mar 30.

Department of Clinical Immunology, School of Medical Laboratory, Tianjin Medical University, Tianjin, China.

Microscopic polyangiitis (MPA) is a systemic autoimmune disease that primarily affects the small and medium blood vessels. Endothelial injury is one of the pathological hallmarks of MPA. However, the pathogenesis for this has not yet been fully elucidated. Read More

Mediterr J Rheumatol 2019 Mar 28;30(1):44-47. Epub 2019 Mar 28.

Rheumatology Department of Asklepieion Voulas General Hospital, Athens, Greece.

We present a case of a 75-year-old woman who admitted in the internal medicine department for a recent onset of persisting moderate daily fever and fatigue that started 30 days prior to her hospitalization. Her past medical history is remarkable for mild pulmonary fibrosis, megaloblastic anaemia, and hypergammaglobulinaemia of no obvious causes. On presentation, she was febrile (38°C) and had high ESR and CRP levels, but most of her laboratory tests were within normal levels and had no signs of arthritis or rash. Read More

CEN Case Rep 2020 Mar 16. Epub 2020 Mar 16.

Division of Nephrology, First Department of Integrated Medicine, Saitama Medical Center, Jichi Medical University, 1-847 Amanuma-cho, Omiya-ku, Saitama, Saitama, 330-8503, Japan.

An 88-year-old man with congenital hemophilia A developed end-stage renal disease due to microscopic polyangiitis. He was at risk for catheter-related infection because he was taking immunosuppressive agents for the treatment of polyangiitis. He was also unable to manipulate the peritoneal dialysis device. Read More

Semin Arthritis Rheum 2020 Feb 24. Epub 2020 Feb 24.

Department of Rheumatology, Santa Chiara Hospital, Trento, Italy.

Objective: Aging may be a risk factor for morbidity in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). We compared the rate and better characterized the type of disease- and treatment-related complications affecting young and elderly patients with AAV.

Methods: All new cases of granulomatosis with polyangiitis or microscopic polyangiitis diagnosed in three referral centers between 2000-2016 were included. Read More

Rev Med Suisse 2020 Mar;16(685):487-491

Service de rhumatologie, Département des spécialités de médecine, HUG, Hôpital Beau Séjour, Avenue de Beau-Séjour 26, 1206 Genève.

Targeted therapies are nowadays commonly used in connective tissue diseases and vasculitis. Experts recommend the use of belimumab and rituximab in refractory and/or severe cases of lupus. Rituximab can be also considered in difficult to treat cases of Sjögren's disease or myositis. Read More

Rheumatology (Oxford) 2020 Mar 11. Epub 2020 Mar 11.

Institute of Infection, Immunity and Inflammation, University of Glasgow, Glasgow, UK.

Objectives: Infection exerts a major burden in ANCA-associated vasculitis (AAV), however, its precise extent and nature remains unclear. In this national study we aimed to longitudinally quantify, characterize and contextualize infection risk in AAV.

Methods: We conducted a multicentre matched cohort study of AAV. Read More

Rheumatology (Oxford) 2020 Mar 9. Epub 2020 Mar 9.

Centre de Référence Maladies Systémiques et Auto-Immunes Rares, Université Paris Descartes, APHP, Hôpital Cochin, Paris, France.

Objective: The randomized, controlled MAINRITSAN2 trial was designed to compare the capacity of an individually tailored therapy [randomization day 0 (D0)], with reinfusion only when CD19+ lymphocytes or ANCA had reappeared, or if the latter's titre rose markedly, with that of five fixed-schedule 500-mg rituximab infusions [D0 + D14, then months (M) 6, 12 and 18] to maintain ANCA-associated vasculitis (AAV) remissions. Relapse rates did not differ at M28. This ancillary study was undertaken to evaluate the effect of omitting the D14 rituximab infusion on AAV relapse rates at M12. Read More

Saudi J Kidney Dis Transpl 2020 Jan-Feb;31(1):182-190

Department of Nephrology, Dialysis and Transplantation, La Rabta Hospital, Tunis, Tunisia.

Glomerulonephritis associated with anti-neutrophil cytoplasmic antibody (ANCA) vasculitis still has a high prevalence of end-stage renal disease (ESRD), particularly in patients with advanced renal failure at presentation. This study aims to evaluate the clinical and histo- pathological features of renal involvement and investigate factors associated with ESRD. Patients with renal biopsy-proven ANCA-associated glomerulonephritis were included retrospectively over a period of nine years (June 2007 to March 2016). Read More

J Community Hosp Intern Med Perspect 2020 10;10(1):50-54. Epub 2020 Feb 10.

Internal Medicine, Liaquat National Hospital and Medical College, Karachi, Pakistan.

Microscopic polyangiitis (MPA) is a primary systemic vasculitis characterized by inflammation of small-sized vessels associated with the presence of anti-neutrophilic cytoplasmic antibodies. We report a case of a 39-year-old female diagnosed with microscopic polyangiitis as an incidental finding who presented with signs and symptoms of a stroke at a young age. Usually, it presents with fever, malaise, skin rash, weight loss, mononeuritis multiplex, and arthralgia/myalgia. Read More

Clin Rheumatol 2020 Jun 27;39(6):1929-1934. Epub 2020 Feb 27.

Faculty of Medicine, Department of Rheumatology, Izmir Katip Celebi University, Karabaglar, 35360, Izmir, Turkey.

Splenic involvement is rarely reported in patients with ANCA-associated vasculitides (AAVs), particularly in those with granulomatosis with polyangiitis (GPA) and is in fact considered to be underestimated. We aimed to investigate the frequency of splenic lesions-mainly infarction-and related factors in patients with AAVs. Patients with AAV whose abdominal or thoracic computed tomographies (CTs) were performed at or after diagnosis were included in the study. Read More

Eur Cytokine Netw 2019 Dec;30(4):151-159

Medical University in Wrocław, Department and Clinic of Otolaryngology, Head and Neck Surgery, Borowska 213 Street, WrocławPoland.

Background: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is an autoimmune disorder of unknown etiology with dysregulated cytokines levels.

Objectives: The main aim of this study was to assess the clinical correlation between antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, granulomatosis with polyangiitis (GPA) serum levels of the microscopic polyangiitis (MPA), serum levels of the proinflammatory cytokines, interleukin (IL)-32 and interleukin-6.

Methods: Study included 71 patients, 47 with GPA and 24 with MPA. Read More

Ocul Immunol Inflamm 2020 Feb 20:1-6. Epub 2020 Feb 20.

Department of Ophthalmology & Visual Science, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan.

ABSTARCT: To investigate ocular manifestations in patients positive for serum anti-neutrophil cytoplasmic antibodies (ANCAs) in Japan.: The clinical records of patients who had ocular manifestations and who were serum ANCA positive between 2011-2017 at Tokyo Medical and Dental University Hospital were retrospectively reviewed.: Eighteen patients were identified to be positive for serum ANCA and had ocular manifestations, including optic nerve involvement (50%), scleritis (27. Read More

Autoimmun Rev 2020 Apr 15;19(4):102495. Epub 2020 Feb 15.

Norwich Medical School, University of East Anglia, Norwich, United Kingdom.

Objective: Renal involvement in anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis is associated with significant morbidity and higher mortality rates. This study examined clinical manifestations associated with renal involvement in ANCA-associated vasculitis within a large, international cross-sectional cohort.

Methods: Univariate and multivariate analyses were performed to identify clinical factors associated with renal disease, which was defined as i) a serum-creatinine >30% above normal and a fall in creatinine-clearance >25%; or ii) haematuria attributable to active vasculitis. Read More

J Clin Rheumatol 2019 Apr 23. Epub 2019 Apr 23.

UERJ (Licensed), Global Medical Expert, Immunology and Specialty Pharma, GSK, Collegeville, PA.

Background/objective: The epidemiology of vasculitis is variable in different geographic areas, and this issue has not been approached in Brazil yet. The objective of this study was to assess the frequency of vasculitis in specialized centers in Brazil.

Methods: This cross-sectional study was performed in 9 vasculitis outpatient clinics from 6 different states mainly from the Southeast and the Northeast regions of Brazil between 2015 and 2017. Read More

Eur J Intern Med 2020 Apr 29;74:18-28. Epub 2020 Jan 29.

Autoimmune Diseases Unit, Internal Medicine Department, Centro Hospitalar de Trás-os-Montes e Alto Douro, Avenida da Noruega, 5000-508 Vila Real, Portugal; NEDAI/SPMI Autoimmune Diseases Group/Portuguese Society of Internal Medicine, Portugal. Electronic address:

Vasculitis is characterized by inflammation of the vascular wall. It reaches vessels of different sizes and locations, conditioning multisystem and complex manifestations that require a holistic approach. Antineutrophil cytoplasmic antibody-associated vasculitis has an annual incidence rate of 20 per million inhabitants. Read More

Case Rep Rheumatol 2019 31;2019:2673947. Epub 2019 Dec 31.

Department of Clinical Immunology and Allergy, Royal North Shore Hospital, Sydney, NSW, Australia.

Background: Microscopic polyangiitis (MPA) can manifest with atypical features such as pulmonary fibrosis and chronic obstructive pulmonary disease (COPD), which are atypical and unusual features of small vessel vasculitis.

Case Presentation: This paper presents two patients with microscopic polyangiitis and respiratory symptoms attributable to atypical pulmonary manifestations. Pulmonary fibrosis was present in both cases, with COPD also present in one patient. Read More

Adv Med Sci 2020 Mar 17;65(1):156-162. Epub 2020 Jan 17.

2nd Department of Internal Medicine, Jagiellonian University Medical College, Krakow, Poland. Electronic address:

Purpose: The aim of this study is to present the treatment modalities and associated side effects in a Polish nation-wide ANCA-associated vasculitides (AAV) patients' cohort.

Materials And Methods: Retrospective analysis of patients diagnosed with AAV between 1990 and 2016, included in the POLVAS registry was performed. Standard descriptive statistic methods were used with an emphasis on the treatment modalities. Read More

Dtsch Med Wochenschr 2020 01 8;145(1):40-46. Epub 2020 Jan 8.

Klinik für Rheumatologie und Klinische Immunologie, Department Innere Medizin, Medizinische Fakultät, Universitätsklinikum Freiburg, Freiburg.

Granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) are the most frequent primary necrotizing small vessel vasculitides. In these formerly fatal diseases remission can be induced by stage- and activity-adapted immunosuppressive regimens in the majority of patients. This does not lead to drug-free long-term remission or even cure. Read More

Pharmacol Res Perspect 2020 02 3;8(1):e00555. Epub 2020 Jan 3.

Joanne Bangs Ltd Letchworth Garden City Hertfordshire UK.

This study used retrospective chart review and survey data to evaluate: (1) off-label use of rituximab (MabThera/Rituxan) in autoimmune conditions and (2) patients' receipt and knowledge of the Patient Alert Card (PAC), a risk minimization measure for progressive multifocal leukoencephalopathy (PML) and serious infections. Anonymized patient data were collected from infusion centers in Europe from December 2015 to July 2017. Adults receiving rituximab in the same centers were provided a self-administered survey. Read More

Clin Dermatol 2019 Sep - Oct;37(5):528-547. Epub 2019 Jul 17.

The Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, New York, USA. Electronic address:

The color purple can be seen in several types of eruptions including inflammatory dermatoses like lichen planus, infectious dermatoses like ecthyma gangrenosum, neoplasms like Kaposi sarcoma, and vasculitis and vasculopathy. The current review focuses on the clinical appearance, pathophysiology, and treatment of several vasculitides and vasculopathies including capillaritis, cutaneous small-vessel vasculitis, immunoglobulin A (IgA) vasculitis, cryoglobulinemia, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, microscopic polyangiitis, polyarteritis nodosum, warfarin-induced skin necrosis, heparin-induced thrombocytopenia, purpura fulminans, antiphospholipid antibody syndrome, calciphylaxis, levamisole-induced vasculopathy, and thrombotic thrombocytopenic purpura. Dermatologists play a central role in treating patients with cutaneous vasculitis and vasculopathy and may have the opportunity to facilitate identification of systemic disease by diagnosing cutaneous vasculitis and vasculopathy. Read More

Front Immunol 2019 6;10:2855. Epub 2019 Dec 6.

Rheumatology Unit, Division of Rheumatology, Allergy, and Immunology, Boston, MA, United States.

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a small- to medium-vessel necrotizing vasculitis responsible for excess morbidity and mortality (1). The AAVs, which include granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA), are among the most difficult types of vasculitis to treat. Although clinicopathologic disease definitions have been used traditionally to categorize patients into one of these three diagnoses, more recently ANCA specificity for either proteinase 3 (PR3) or myeloperoxidase (MPO) has been advocated for the purpose of disease classification (2). Read More

Mod Rheumatol 2020 Jan 6:1-9. Epub 2020 Jan 6.

Okayama University, Okayama, Japan.

We evaluated the effectiveness of cyclophosphamide for patients with microscopic polyangiitis and granulomatosis with polyangiitis. Patients treated with cyclophosphamide and glucocorticoid (cyclophosphamide group) or glucocorticoid alone (non-cyclophosphamide group) for remission-induction were enrolled from two Japanese nationwide prospective inception cohort studies. The effectiveness and safety outcomes were compared before and after propensity score (PS)- matching. Read More

Rheumatology (Oxford) 2019 Dec 20. Epub 2019 Dec 20.

Department of Rheumatology, University Hospital of Northern Norway, Tromsoe, Norway.

Objective: ANCA-associated vasculitides (AAV) have increased in prevalence since the 1980s. We aimed to investigate the incidence and prevalence of AAV during a 15-year period from 1999 to 2013 in Northern Norway, looking for variations during this period.

Methods: Patient records were retrieved from The Northern Norwegian Vasculitis Registry; in addition we searched all regional hospital databases. Read More

Semin Arthritis Rheum 2019 Nov 15. Epub 2019 Nov 15.

Department of Rheumatology, Hanyang University Hospital for Rheumatic Diseases, 222, Wangsimni-ro, Seongdong-gu, Seoul, Republic of Korea, 04763, South Korea. Electronic address:

Objectives: To estimate the prevalence, medical utilization, and recent changes in the economic burden of autoimmune rheumatic diseases (AIRDs) in Korea.

Methods: Using a nationwide claims database that includes all medical claims made by approximately 50 million Korean residents, the prevalences of seropositive rheumatoid arthritis (RA), ankylosing spondylitis (AS), systemic lupus erythematosus (SLE), and others between 2012 and 2016 were calculated. Changes in medical utilization and the direct medical costs of each AIRD from 2012 to 2016 were also evaluated. Read More

Clin Immunol 2020 Feb 11;211:108325. Epub 2019 Dec 11.

Departments of Pediatrics and Medicine, University of Toronto, Division of Rheumatology, The Hospital for Sick Children, Toronto, ON, Canada. Electronic address:

Antineutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV) is a term used to describe rare primary systemic vasculitides affecting small and medium-sized blood vessels. AAV diseases which include Granulomatosis with Polyangiitis (GPA), Eosinophilic Granulomatosis with Polyangiitis (EGPA), Microscopic Polyangiitis (MPA) and renal limited ANCA vasculitis. These multisystemic disorders involve upper and lower respiratory tract and kidneys associated with organ damage and long term sequelae. Read More

Rheumatology (Oxford) 2020 01;59(1):77-83

Department of Internal Medicine, Referral Center for Rare Systemic and Autoimmune Diseases, Hôpital Cochin, APHP, Université Paris-Descartes, Paris Cedex 14, France.

Objective: To identify the role of Staphylococcus aureus (SA) or its intracellular small-colony variant phenotype (SCV) with co-trimoxazole (CTX) or ANCA-associated vasculitis (AAV) activity.

Methods: All consecutive AAV patients with granulomatosis with polyangiitis (GPA), eosinophilic GPA or microscopic polyangiitis, followed at the French National Vasculitis Referral Center (09/2012-05/2013), and hospitalized non-AAV controls, exclusively for SA/SCV carriage comparisons, were enrolled. All had bilateral anterior nasal swab cultures. Read More

Clin Chim Acta 2020 Feb 2;501:12-19. Epub 2019 Dec 2.

Renal Division, Department of Medicine, Peking University First Hospital, Peking University Institute of Nephrology, Key Laboratory of Renal Disease, Ministry of Health of China, Key Laboratory of Chronic Kidney Disease Prevention and Treatment (Peking University), Ministry of Education, Beijing 100034, China; Peking-Tsinghua Center for Life Sciences, Beijing 100034, China.

Background: We investigated the characteristics of antineutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis (ANCA-GN) and immunoglobulin G4 (IgG4)-related kidney disease (IgG4-RKD) overlap syndrome.

Methods: This is a 2-center study with 19 patients.

Results: Fifteen patients were classified as microscopic polyangiitis (MPA). Read More

J Rheumatol 2019 Dec 1. Epub 2019 Dec 1.

From the Department of Pulmonology, Hôpital Foch, Suresnes, Faculté des Sciences de la Vie, Simone Veil, Université de Versailles, UPRES EA 220, France; Sorbonne Université, UPMC, Pierre Louis Institute of Epidemiology and Public Health (IPLESP UMRS 1136), Epidemiology of Allergic and Respiratory Diseases Department (EPAR), Saint-Antoine Medical School, Paris; Department of Internal Medicine, Orléans, France; Department of Internal Medicine, CHU, Tours, France; Department of Internal Medicine, CEREO (National Referral Center for Hypereosinophilic Syndromes), Hôpital Foch, Suresnes, France; Department of Internal Medicine, National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, AP-HP, Université Paris Descartes, Paris; Department of Internal Medicine, Saint Eloi hospital, Montpellier, France; 8Sorbonne Université, INSERM UMRS 1135, Department of Internal Medicine 2, Centre National de Référence Maladies Auto-Immunes et Systémiques Rares Lupus et Syndrome des Anticorps Antiphospholipides Centre de Référence des Histiocytoses, Institut E3M, AP-HP, Hôpital Pitié-Salpêtrière, Paris, France; Department of Pulmonology , Louis Pradel hospital, Lyon, France; Departments of Internal Medicine, University Hospital, Caen, France; Department of Internal Medicine, François Mitterand hospital, Pau, France; Department of Pulmonology department, Charles Nicolle University hospital, Rouen, France; Department of Internal Medicine, University Hospital, Amiens, France; Department of Internal Medicine, Saint Antoine hospital, Paris, France; Department of Internal Medicine, Centre de Reference des Maladies Auto-immunes Systémique Rares du Nord et du Nord-Ouest de France (CeRAINO), CHRU de Lille, Université de Lille, Lille, France; Department of internal medicine, CHU, Nantes, France; Department of Nephrology, Necker Enfants Malades hospital, Paris, France, Department of Internal Medicine, Delafontaine hospital, Saint Denis, France; Department of Internal medicine, Dax hospital, Dax, France; Department of Pulmonology , Avicenne hospital, Bobigny, France; Department of Nephrology-Dialysis-Transplantation, Angers University Hospital, Angers, France; Department of Radiology, Avicenne hospital, Bobigny, France. Corresponding author: Dr Colas Tcherakian, Service de Pneumologie, hôpital Foch, 40 rue Worth, 92150, Suresnes, France; E-mail:

Objective: To report on a large series of patients with antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) and bronchiectasis, with a specific focus on the timeline of occurrence of both features.

Methods: Retrospective nationwide multicentric study of patients diagnosed with both AAV and bronchiectasis.

Results: Sixty-one patients were included among which 27 (44. Read More

Expert Rev Clin Immunol 2019 12 24;15(12):1273-1286. Epub 2019 Nov 24.

Division of Pulmonary and Critical Care Medicine, Mayo Clinic College of Medicine and Science, Rochester, MN, USA.

: The majority of the patients with anti-neutrophil cytoplasmic autoantibody (ANCA) associated vasculitis (AAV) achieve remission with effective induction therapy. Therefore, prevention of relapses and avoiding long-term damage and treatment-related toxicity are major challenges.: This review provides an update on maintenance therapy in AAV, emphasizing the available treatment options for granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). Read More